Suprasellar Hemangioblastoma Unrelated to von Hippel-Lindau Disease Successfully Treated through Extended Transsphenoidal Approach: Diagnostic Value of Von Hippel-Lindau Disease Gene-Derived Protein

2016 ◽  
Vol 78 (03) ◽  
pp. 296-301 ◽  
Author(s):  
Jia Wenting ◽  
Jun Ito ◽  
Teiji Tominaga ◽  
Yoshikazu Ogawa
Keyword(s):  
Disease Gene ◽  
Diagnostic Value ◽  
Transsphenoidal Approach ◽  
Von Hippel Lindau ◽  
Extended Transsphenoidal Approach ◽  
Von Hippel Lindau Disease

Molecular Analysis of the von Hippel-Lindau Disease Gene

2001 ◽  
pp. 193-216
Author(s):  
Allen Chernoff ◽  
Viera Kasparcova ◽  
W. Marston Linehan ◽  
Catherine A. Stolle
Keyword(s):  
Molecular Analysis ◽  
Disease Gene ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

scholarly journals Somatic mutations of the von Hippel-Lindau disease gene in renal carcinomas occurring in patients with long-term dialysis

2007 ◽  
Vol 22 (7) ◽  
pp. 2052-2055 ◽  
Author(s):  
H. Inoue ◽  
N. Nonomura ◽  
Y. Kojima ◽  
M. Shiba ◽  
D. Oka ◽  
...  
Keyword(s):  
Somatic Mutations ◽  
Disease Gene ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease

scholarly journals Progressive peritumoral edema defining the optic fibers and resulting in reversible visual loss

2008 ◽  
Vol 109 (2) ◽  
pp. 313-317 ◽  
Author(s):  
Martin Baggenstos ◽  
Emily Chew ◽  
John A. Butman ◽  
Edward H. Oldfield ◽  
Russell R. Lonser
Keyword(s):  
Color Vision ◽  
Vision Loss ◽  
Tumor Resection ◽  
Vascular Tumor ◽  
Peritumoral Edema ◽  
Von Hippel Lindau ◽  
Extended Transsphenoidal Approach ◽  
Von Hippel Lindau Disease ◽  
Optic Fibers ◽  
Reversible Visual Loss

Hemangioblastomas are frequently associated with peritumoral edema caused by extravasation of plasma ultrafiltrate through permeable neoplastic vessels. The authors report the clinical and imaging findings in a 62-year-old man with von Hippel–Lindau disease who presented with rapid (within 24 hours) loss of color vision and nearcomplete loss of left eye vision (acuity too poor to test). Serial MR imaging demonstrated a stable vascular tumor in the medioinferior aspect of the left optic nerve, associated with progressive edema extending from the nerve through to the bilateral optic radiations. Complete resection of the lesion was performed via an extended transsphenoidal approach, and histological examination confirmed the lesion was a hemangioblastoma. Postoperatively, the patient recovered color vision and had improvement in visual acuity (20/320). Serial imaging in this unique case captured the progressive extravasation of peritumoral edema that tracked and defined the parallel white matter tracts of first- and second-order neurons of the optic system, causing vision loss. Tumor resection led to resolution of the edema and improvement in visual function.

scholarly journals Medullary hemangioblastoma in a child with von Hippel-Lindau disease: vascular tumor perfusion depicted by arterial spin labeling and dynamic contrast-enhanced imaging

2015 ◽  
Vol 16 (1) ◽  
pp. 50-53 ◽  
Author(s):  
Hyun Woo Goo ◽  
Young-Shin Ra
Keyword(s):  
Arterial Spin Labeling ◽  
Vascular Tumor ◽  
Spin Labeling ◽  
Diagnostic Value ◽  
Tumor Perfusion ◽  
Von Hippel Lindau ◽  
Dynamic Contrast Enhanced Imaging ◽  
Dynamic Contrast Enhanced ◽  
Contrast Enhanced ◽  
Von Hippel Lindau Disease

Medullary hemangioblastoma is very rare in children. Based on small nodular enhancement with peritumoral edema and without dilated feeding arteries on conventional MRI, hemangioblastoma, pilocytic astrocytoma, oligodendroglioma, and ganglioglioma were included in the differential diagnosis of the medullary tumor. In this case report, the authors emphasize the diagnostic value of arterial spin labeling and dynamic contrast-enhanced MRI in demonstrating vascular tumor perfusion of hemangioblastoma in a 12-year-old boy who was later found to have von Hippel-Lindau disease.

Sign in / Sign up

Export Citation Format

Share Document